Female hypospadias leading to delayed end stage renal disease - prenatal and neonatal correlation
Presentation
Isolated urinary anomaly detected by antenatal ultrasound. Lack of neonatal follow-up from day 3 until 5 months, when presented with hemodynamic shock and hyperkalemia complicated by cardiac arrest and requiring emergency hemodialysis. At ER, parents reported that urination had been impossible for 3days.
Patient Data
Prenatal US from 11 to 35...
Prenatal US from 11 to 35 wk gestational age
Age of gestation (AG) is shown on the top left corner.
Postnatal US D3 pre-discharge
US performed at day of life 3 showed right hydroureteronephrosis, and mild pyelic dilation on the left side. Bladder was full, and ureteral urine stream was visible on color Doppler.
Postnatal Uro-MRI at 5 mth...
Postnatal Uro-MRI at 5 mth while in ICU for renal failure
MRI revealed bilateral megaureters with renal parenchymal thinning. No other associated genital or neurologic anomaly.
Anterograde cystography...
Anterograde cystography through suprapubic catheter lower urinary tract catherization failure
Bladder catheterization was impossible, hence the pediatric surgeon had to use a suprapubic cathether. Anterograde cystography through a suprapubic catheter revealed an urethral abnormal tract leading to vaginal opacification.
Case Discussion
Clinical examination revealed an abnormal urethral meatus on the anterior vaginal wall, consistent with female hypospadias.
This case demonstrates the classic delayed clinical course of female hypospadias leading to obstructive renal failure. The prenatal findings of renal pelviectasis are mild and nonspecific, and yet the later presentation was dramatic and critical.
Female hypospadias, defined as a female urethra opening on the anterior vaginal wall, is a sneaky congenital urological condition. It is hard to diagnose clinically, and frequently asymptomatic until end-stage renal disease (ESRD) due to lower urological tract obstruction. There have been case reports of female hypospadias diagnosed in teenagers at ESRD.
To our knowledge, this is the first report of prenatal and postnatal correlation.
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